1. Making the diagnosis in Hematology Part 2

2. Myeloproliferative disorders

Polycythaemia

Essential thrombocythaemia

Myelofibrosis

Myelodysplasia.

3. Polycythaemia

Absolute erythrocytosis.

Primary

Secondary

Relative polycythaemia (Gaisbocks syndrome)

4. Causes of secondary Polycythaemia 5. Diagnosis of PV 6. Features suggestive of Polycythaemia?

Hypertension

Angina

Intermittent claudication

Tendency to bleed

Severe itching after a hot bath .

Gout

Peptic ulceration.

7. Lab features to distinguish between primary and secondary polycythaemia

InPrimary ,low serum EPO .

In secondary, EPO levels normal or raised.

8. Features of relative polycythaemia(Gaisbock's syndrome)

An apparent rise of the erythrocyte level in the blood.

Normal red cell volume .

Reduced plasma volume .

Often caused by loss of body fluids, such as throughburns, dehydration and stress .

9. Common scenario for relative polycythaemia

Middle-aged men

Smokers

Obese

Hypertensive.

May present with cardiovascular problems such as myocardial or cerebral ischaemia.

10. Essential thrombocythaemia

Patients havenormal Hb levels and WBCbutelevated platelet counts.

At diagnosis the platelet count will usually be 600 10 9 /L, and may be as high as 2000.

As a general rule, if a person who is clinically normal with good health has a very high platelet count (> 1000 109/L) will most likely prove to have ET.

JAK2 mutation in 50% of patients.

11. Myelofibrosis

Blood film: Poikilocytesand red cells with characteristictear-dropforms are seen.

The WBC >100.

High platelet count may be very high, but in later stages, thrombocytopenia occurs.

Bone marrow aspiration oftenunsuccessful.

Abone marrow trephineis necessary to show the markedlyincreased fibrosis .

12.

Increased numbers of megakaryocytes may be seen.

The Philadelphia chromosome isabsent.

The leucocyte alkaline phosphatase(LAP)score isnormal or high .

High serum urate is present.

Low serum folate levels

13. Lab investigation to help distinguish between myelofibrosis vs CML

Appearance of the bone marrow and theabsence of the Philadelphia chromosomein myelofibrosis.

14. Myelodysplasia

Pancytopenia:

anaemia

neutropenia

monocytosis

Thrombocytopenia

bone marrowusuallyshows increased cellularitydespite the pancytopenia.

Ring sideroblastsare present in all types.